儿童急性早幼粒细胞白血病早期死亡危险因素分析

Analysis of risk factors for early death of acute promyelocytic leukemia in children

目的分析初诊急性早幼粒细胞白血病(APL)早期死亡患儿的临床特点,探讨直接死亡原因和导致早期死亡的危险因素。方法回顾性分析2013年1月至2020年12月连续收治的初诊APL患儿的临床资料。结果纳入103例APL患儿,男56例、女47例,中位发病年龄9.0(7.0~13.0)岁,低、中、高危组患儿分别为24例、41例、38例,早期死亡13例。与生存组比较,早期死亡组初诊白细胞计数(WBC)≥100×109/L、凝血酶原时间(PT)延长≥3 s、D-二聚体≥9 mg/L、骨髓早幼粒细胞≥90%、危险度为高危的比例较高,差异均有统计学意义(P<0.05)。发生早期死亡的13例患儿中,男10例、女3例,从诊断至死亡的中位时间为4.0(2.0~7.0)d。13例患儿均死于出血,其中颅内出血13例,颅内出血合并肺出血5例。多因素logistic回归分析显示,初诊WBC≥10×109/L(OR=17.27,SE=1.12,95%CI:1.92~155.04)是发生早期死亡的独立危险因素。结论儿童APL早期病死率高,初诊WBC≥10×109/L是APL患儿发生早期死亡的重要预测因子。

Objective To analyze the clinical characteristics of early death in newly diagnosed children with acute promyelocytic leukemia(APL),and to explore the direct causes of death and the risk factors leading to early death.Methods The clinical data of newly diagnosed APL children admitted from January 2013 to December 2020 were retrospectively analyzed.Results A total of 103 children with APL(56 boys and 47 girls)were included.The median age of onset was 9.0(7.0-13.0)years old.There were 24,41 and 38 children in low,medium and high risk groups,respectively.Early death occurred in 13 children.Compared with the survival group,the early death group had higher proportions of initial white blood cell count(WBC)≥100×109/L,prothrombin time(PT)≥3 s,D-dimer≥9mg/L,bone marrow promyelocytes percentage≥90%,and high risk,and the differences were statistically significant(P<0.05).Among the 13 children who died early,10 were boys and 3 were girls.The median time from diagnosis to death was 4.0(2.0-7.0)days.All the 13 children died of hemorrhage,including 13 cases of intracranial hemorrhage and 5 cases of intracranial hemorrhage combined with pulmonary hemorrhage.Multivariate logistic regression analysis showed that WBC≥10×109/L at first diagnosis(OR=17.27,SE=1.12,95%CI:1.92-155.04)was an independent risk factor for early death.Conclusions The early case fatality rate of children with APL is high,and WBC≥10×109/L at first diagnosis is an important predictor of early death in children with APL.